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Classification of Systemic Vasculitis

Vasculitis may accompany a number of different diseases (secondary systemic vasculitis, SSV). Besides a paraneoplastic or infection-associated immuno-pathogenesis, direct invasion of vessels by infectious organisms or by tumor cells can lead to vasculitis. In addition, secondary vasculitis occurs in association with connective tissue disease or as drug-induced vasculitis. (tables 3- 5).

Table 3: Infection-Associated Vasculitis

Leukocytoclastic vasculitis including Schoenlein-Henoch's purpura
Bacterial: streptococci, staphylococci, salmonella, yersinia, mycobateriae
Viral: varicella-zoster, hepatitisvirus B, C, cytomegalovirus, influenza virus
Panarteritis nodosa
Bacterial: streptococci
Viral: hepatitisvirus A,B,C, HIV, parvovirus
Kawasaki's syndrome
Bacterial: streptococci, samonella, yersinia, mycoplasma
Viral: parainfluenza, rotavirus, HHV-6

 

Table 4: Neoplasia-Associated Vasculitis

NeoplasiaType of Vasculitis
Non-Hodgkin's lymphoma
Hodgkin's disease
Myelogenous leukaemia
Adenocarcinoma
Carcinoma of the bronchus
granulomatous, lymphocytic, leukocytoclastic, or necrotizing
leukocytoclastic
granulomatous, leukocytoclastic, or necrotizing
leukocytoclastic
leukocytoclastic
( see also lymphomatoid granulomatosis: table 5)

Table 5: Vasculitis Accompanying Connective Tissue Diseases

EntityType of Vasculitis
SLE Systemic Lupus Erythematosus lymphocytic proliferative vasculitis &
perivasculitis with concentric fibrosis
("onion skin")
Figure 10: butterfly examthema
Figure 11: exanthema, dorsum
Figure 12 & Figure 13: membranoproliferative glomerulitis (immuncomplex-glomerulitis)
RARheumatoid Arthritissmall vessel vasculitis with intimal
proliferation, little cellular infiltrate
& immune deposits, early fibrinoid necrosis
fibrosis of vessel wall
Figure 14: vasculitis in rheumatoid arthritis
Figure 15: rheumatoid granuloma with fibrinoid necrosis
Figure 16 & Figure 17: rheumatoid synovitis
PSS Primary Systemic Sclerosis (Scleroderma) proliferating (intimal) and sclerosing vasculitis
early changes: Raynaud's
Figure 18 & Figure 19: dermal atrophy, sclerosis and ulceration
Figure 20 & Figure 21: dermal histology, sclerosis and degeneration of elastic membranes
Figure 22: concentric sclerosis of vessels; here in kidney
SS Sjogren's syndrome small & medium sized vessels, variable:
leukocytoclastic, mononuclear or mixed
Figure 23: lymphocytic sialoadenitis, lip biopsy
Figure 24: pseudolymphomatous sialoadenitis (progressing to lymphoma)
UCTDUnclassified Connective Tissue Diseaseany of above